Symptoms and Signs of Childhood Atypical Teratoid/Rhabdoid (Brain Tumor)

Medical Author:
Medically Reviewed on 9/30/2021

Doctor's Notes on Childhood Atypical Teratoid/Rhabdoid (Brain Tumor)

Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor that occurs when cells in the tissues of the brain and spinal cord start to grow abnormally. It usually occurs in children aged 3 and younger, although it can occur in older children and adults. About half these tumors form in the:

  • cerebellum, the part of the brain that controls movement, balance, and posture, or
  • brain stem, the part of the brain that controls breathing, heart rate, and
  • the nerves and muscles used in seeing, hearingwalking, talking, and eating. 

Symptoms of central nervous system (CNS) atypical teratoid/rhabdoid tumor depend on the child's age and where the tumor formed. Atypical teratoid/rhabdoid tumors are fast-growing, so symptoms may develop quickly and worsen over a period of days or weeks. Symptoms of AT/RT may include: 

  • morning headache or headache that goes away after vomitingnausea and vomiting,
  • unusual sleepiness or change in activity level,
  • loss of balance,
  • lack of coordination,
  • trouble walking, or
  • an increase in head size (in infants).

What Is the Treatment for Childhood Atypical Teratoid/Rhabdoid Tumor?

Treatment for CNS atypical teratoid/rhabdoid tumor in children depends on the age of the child and the amount of tumor remaining after surgical removal. In general, several treatments may be used:

Clinical trials are also underway to test new therapies, including targeted therapies. Targeted therapies are drugs that have been developed to attack tumor cells that contain specific genetic or molecular changes.


Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.